Abstract

Malignant fibrous histiocytoma (MFH) occurring in the heart is rare. To the best of our knowledge, the current study was the first to report a case of left atrial MFH metastasizing to the vulva. A 37-year-old female presenting with a history of exertional dyspnea underwent echocardiography, which revealed a mass in the left atrium, with a short-wide stalk attached to the posterior wall, extending into the pericardium. Furthermore, the mass resulted in severe mitral stenosis during diastole. The tumor was excised together with a section of the left atrial wall. The initial diagnosis was a left atrial myxoma. However, post-operative frozen section histopathology results indicated a diagnosis of MFH. Following surgery, the patient received chemotherapy, however, vulvar metastases and left pleural effusions were identified after 1 month. Following surgical excision of the vulvar tumor and left thoracentesis, the patient began adjuvant chemotherapy. However, the patient succumbed due to local recurrence 6 months later.

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